An Opportunity Missed: Managing an Aggressive Adrenal Cortical Carcinoma

Adrenal cortical carcinoma (ACC) is a rare endocrine tumor with an incidence of up to 2 persons per million population. Complete surgical resection is the first-line therapy for localized tumors, while debulking surgery, radiation therapy, and/or chemotherapy may also be options for palliation in advanced ACC. Biochemical and radiographic evaluation of a newly discovered adrenal mass maximizes the opportunity for the patient to undergo a complete, margin-negative resection of the primary tumor, which is the most powerful determinate of long-term survival. Here we present the case of an extremely aggressive high grade adrenal cortical carcinoma that increased from 4 cm to 17 cm within 4 months, metastasizing to the lung, liver and left kidney prior to resection. Case summary: A 42-year-old woman presented to the Emergency Department (ED) with abdominal pain and was found to have hypokalemia and an incidental left adrenal mass measuring 4.9 x 4.6 cm. She was discharged home with instructions to follow up with her primary care physician for further workup. Unfortunately, she was lost to follow-up for 3 months. Her initial biochemical evaluation at follow-up did not show evidence of adrenal insufficiency or elevated metanephrines. A repeat outpatient CT chest abdomen pelvis revealed increased tumor size to 12.3 x 10.8 x 12.5 cm, with unclear renal vs adrenal origin, and multiple liver and lung metastases. She subsequently underwent left radical nephrectomy, adrenalectomy, splenectomy as well as a partial diaphragmatic resection 4 months after her initial ED presentation. The surgical specimen measured 17 x 11.5 x 8 cm. Endocrinology was consulted post-operatively to evaluate for relative adrenal insufficiency given hypotension & altered mental status. Further evaluation revealed new-onset hirsutism, male-pattern hair loss, proximal muscle weakness, and significant weight loss over the prior 3 months. Post-operative laboratory evaluation was significant for sustained hypokalemia of 2.4 mmol/L, 24 hour urine cortisol of 1326 mcg/24 hr (normal 3.5–45), as well as elevated DHEA-S of 994 mcg/dL (normal 27–240), and testosterone of 294 ng/dL (normal 10–75). Surgical pathology demonstrated a high-grade adrenal cortical carcinoma. Oncology was consulted and the patient was started on spironolactone for the hypokalemia, but declined any further therapy for her ACC and subsequently died 2 weeks later. Conclusion: This case illustrates how ACC can behave aggressively and further emphasizes the need for timely and complete biochemical evaluation of adrenal incidentalomas. This is particularly true with the presence of suspicious radiologic features such as size more than 4 cm and heterogeneous appearance. All patients with an adrenal incidentaloma should undergo biochemical and imaging evaluation, guided by thorough history-taking and physical exam, to ensure optimal outcomes.